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6. TSE and rabies
use for Exam 2 too
83
Veterinary Medicine
Professional
02/05/2016

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Term
What does TSE stand for?
Definition
-transmissible spongiform encephaloptathy
Term
What agents cause TSEs?
Definition
-prion
Term
Can you see prions under EM?
Definition
-no
Term
Are prions resistant or susceptible?
Definition
-extremely resistant to everything: UV, ionizing radiation, boiling for 30 mins, proteases, nucleases, and formaldehyde
Term
True or False: Prions are a class of viruses.
Definition
-FALSE, prions are NOT viruses
Term
What is/are the affected species of Scrapie? Source of infection?
Definition
-sheep
-placenta, blood, fetal tissues
Term
What is/are the affected species of Bovine Spongiform Encephalopathy (BSE)? Source of infection?
Definition
-cattle
-prion contaminated meat and bone meal
Term
What is/are the affected species of Transmissible Mink Encephalpathy? Source of infection?
Definition
-mink
-prion contaminated meat
Term
What is/are the affected species of Chronic Wasting Deases (CWD)? Source of incetion?
Definition
-mule, deer, elk
-feces, placenta
Term
What is/are the affected species of Feline Spongiform Encephaloptahy? Source of infection?
Definition
-cats, zoo fleids
-BSE-contaminated meat
Term
What is/are the affected species of Exotic Ungulate Spongiform Encephalopathy? Source of infection?
Definition
-kudu, nyala
-BSE-contaminated meal
Term
What is/are the affected species of Atypical Scrapie and BSE? Source of infection?
Definition
-sheep, cattle
-Sporadic origin
Term
What is/are the affected spp of Kuru? Source of infection?
Definition
-humans
-cannabalism
Term
What is/are the affected species of Creuzfeld-Jakob Disease (CJD)? Source of infection?
Definition
-humans
-BSE-contaminated meat
Term
How do prions replicate?
Definition
-prion proteins PrP(Sc) convert normal cellular proteins PrP(C) to rogue prion proteins
Term
Where doe PrP^C proteins come from?
Definition
-they are normal cellular glycoproteins that form part of the cell membranes of neurons and lymphoid cells
Term
What physical change converts PrP^C to PrP^Sc?
Definition
-the alpha helices are converted into beta sheets
Term
After lots of abnormal rogue PrP^Sc proteins are formed, then what happens?
Definition
-they accumualte, polymerize, and form helical rod-like structures
Term
Describe how an animal can be genetically predisposed to infection with Scrapie.
Definition
-prsence of glutamine at codon 171 in PrP^C gene = susceptible
-those with arganine are not
Term
Describe the incubation of prions.
Definition
-months to years
Term
True or False: Diseases caused by prions are always fatal.
Definition
-TRUE
Term
How are prions transmitted?
Definition
-infected organs or secretions
Term
Where is pathology caused prions geographically restricted to?
Definition
-brain only
Term
Describe the histopathological appearance of prion disease.
Definition
-vacuolation and degeneration of neurons (grey matter), hypertrophy of astrocytes, and by a COMPLETE ABSENCE OF INFLAMMATION
Term
True or False: There is an easily detected host immune respose to prions.
Definition
-FALSE
Term
Describe Scrapie.
Definition
-non-febrile, fatal, crhonic dz of sheep and goats characterized clinically by pruritis (itching and rubbing), tremors, weaving gait and incoordination, and eventual hindquarter paralysis
Term
What is the incubation of scrapie?
Definition
-1-4 y
Term
Which age group of sheep is affected by scrapie? Which breeds are especially susceptible?
Definition
-sheep older than 18m
-Suffolk (black face) breeds
Term
Where geographically does Scrapie occur? How is it naturally transmittted?
Definition
-Europe and N. America
-ingestion and during birth
Term
The sheep most susceptible to Scrapie are those designated ______ and the most resistant are ______. Are heterozygous sheep resistant or susceptible?
Definition
-171-QQ homozygous
-171RR homozygous
-resistant
Term
Outline the progression of clinical signs of Scrapie in sheep.
Definition
-aggression => pruritus (nibbling reflex) => uncoordination => emaciation => hindquarter paralysis
Term
In the US, Scrapie is under the control of which official office? How do they respond to infected farms?
Definition
-USDA
-quarantine and test for genetic susceptibility, then slaughtered or tested for infection
Term
How are sheep genetically tested for Scrapie susceptability?
Definition
-PCR and prion testing on tissue by immunohistochemistry
Term
Bovine Spongiform Encephalotpathy was first recognized when and where?
Definition
-Britain in1986
Term
Describe the clinical signs of BSE
?
Definition
-period of hyper-excitability during which some animals become aggressive and charge people
-incoordination, ataxia, slow progressive paralysis
Term
How did the disease start spreading in 1986? To what other domestic animals did it spread?
Definition
-ifected meat and bone meal fed to cows as protwin supplement
-to cats
Term
Is BSE constant or sporadic?
Definition
-sproadic
Term
The 4th case of BSE in the US was reported on April 12, 2012. How did it happen? How did we fix it?
Definition
-originated from feeding of contaminated cattle feed imported from Britain prior to the ban
-US banned importation of feed from Britain in 1997
Term
How do we screen for BSE?
Definition
-section ofthe brian, brain stem, and spinal cord from slaughtered cattle are sectioned, put on slides, and digested with proteinase K (proteinase K digests PrP^C but not the PrP^Sc) and then treated with monoclonal Ab
Term
How is Transmissible Mink Encephalopathy transmitted?
Definition
-carcasses of sheep infected with Scrapie were fed to minks and it was spread between minks by cannabilism
Term
What does CWD stand for?
Definition
-Chronic wasting disease of mule deer and elk
Term
Where was CWD first noted?
Definition
-Fort Collins, Colorado
Term
How is CWD diagnosed?
Definition
-brain leasions typical of spongiform encephalotpathy
Term
What animals are affected by CWD?
Definition
-free ranging mule deer, black-tailed deer, white-tailed deer, Mountain elk, and Shira's moose
Term
Which animals have the highest incidence of CWD?
Definition
-mule deer
Term
What are the clincial signs of CWD?
Definition
-teeth grinding, abnormal behavior, excessive water intake, and marked loss of weight
Term
What is the source of infection of CWD?
Definition
-prions in the feces
Term
In 1988, a novel form of Scrapie wad identified in Norway. What makes it novel?
Definition
-affected the cerebellum as opposed to the medulla
Term
What are the novel forms of TSE?
Definition
-atypical bovine, ovine, and caprine spongiform encephalopaties
Term
Which animals are affected by novel forms of TSE?
Definition
-older, clinically normal animals
Term
What are a few synonyms of the term "rabies"?
Definition
-Hyrdophobia and La Rage
Term
What are the family and genus of rabies virus?
Definition
-Family: Rhabdoviridae
-Genus: Lyssavirus
Term
How many serotypes of rabies are there? What about strains?
Definition
-one serotype
-many strains
Term
What are the 4 straims of rabies virus?
Definition
1) Street virus strain: refers to field isolates
2) fixed virus: virus that has been passaged and has reached a standard incubation time in that hose (usually less virulent)
3) Flurry strain: strain of human origin
4) Various variants have been isolated from different animal species and countries
Term
Rabies is present on most parts of the world except...
Definition
-Australia, New Zealand, Britain, Hawaii, Seychelles, and other islands
Term
All warm blooded mammals are susceptible but one species is quie resistant. What is it?
Definition
-opossums
Term
What are the 5 principle rabies reservoirs in the US and where is it mostly in each?
Definition
1) Skunks: central states & CA
2) Raccoons: SE
3) Foxes: North and SW
4) BAts: important reservoir throughout the US
5) Coyote: extreme southern region of TX
Term
How is rabies transmitted?
Definition
-bites and inhalation b/n bats
Term
How long is the incubation period of rabies?
Definition
-3w to 5y
Term
Where in the body does rabies virus replciate?
Definition
-muscle fibers in the vicinity of the NMJ near the bite would
Term
How does rabies get into the brain? Where does it finally end up?
Definition
-migrates up the nerve by axoplasm flow to infect the neurons
-virus then spreads centripetally to salivary glands and other organs via nerve cells
Term
Where in the body is rabies excreted? Thus is there a notable viremia?
Definition
-saliva
-NO
Term
True or False: Rabies virus is never present in the saliva before clinical signs of rabies are seen.
Definition
-FALSE, can be in salive before clinical signs are seen
Term
In domestic animals in the US and MX, is rabies usually fatal? What about in the Philippines and Africa?
Definition
-uniformly fatal in US and MX
-can suvive and act as a reservoir in PHilippines and Africa
Term
What are the three forms of rabies?
Definition
1) Prodromal form
2) Furious form
3) dumb or paralytic form
Term
True or False: The one and only way in which rabies progresses is from prodromal form => furious form => paralytic form.
Definition
-FALSE can have one, two, or three forms in any order
Term
What occurs during the prodromal form of raies?
Definition
-change in temperment, slight fever, dilation of pupils, lick the site of bite wound constantly
-horses present with lameness
Term
How does the furious form of rabies present/
Definition
-loos fear of humans
-abnormal aggressive behavior wihtou provocation
-change in voice
-salivation without ability to swallow
-abnormal sexual behavior
-tenesmus with paralysis of tail and anal sphincter msucles
Term
How does the paralytic form of rabies present?
Definition
-progressive ascending paralysis starting with hind legs and spreading anteriorly
-death quickly follows after the onset of paralysis
Term
How does rabies present histopathologically?
Definition
-non-suppurative encephalomyelitis, neuronal degeneration, perivascular cuffing
-negri bodies often seen
Term
What does a Negri body look like?
Definition
-eosinophilic round inclusions in the cytoplasm of neuons in CNS
Term
What are the two most important tests used to diagnose rabies?
Definition
-IFA an d immunoperoxidase
Term
How does the mouse inoculationt est for rabies work?
Definition
-inoculate mice with homogenized tissue intracerebrally then do a FA test on brain of dead or euthanized mice
Term
How do we serologically test man for rabies?
Definition
-look for Ab in CSF
Term
How do we use PCR to test for rabies?
Definition
-look for the presence of genomic RNA and mRNA
Term
Rabies vaccinated animal have which immunoglobulin?
Definition
-VN IgG
Term
Only ___% of people exposed to bites of rabid dog will develop rabies.
Definition
-20%
Term
What do the recombinant rabies vaccine used on wild animals contain?
Definition
-G-protein protein
Term
What is the vaccine protocol for rabies for domestic animals?
Definition
-at 3m, 1y, then yearly or every 3y
Term
If a human is bitten by a rabid dog, what are our options?
Definition
-euthanize immediately and send brain to State lab
-confine animal for 10d and observe
Term
What happens when a vaccinated pet is bitten?
Definition
-give a booster within 5d
Term
What happens when an unvaccinated animal is bitten?
Definition
-euthanize immediately
-quarantine for 6m under vet supervision and vaccinate 3 times
Term
True or False: For post-exposure treatment in vaccinated humans, we give immune globulin.
Definition
-FALSE, do not give immune globulin to persons that have been vaccinated
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